What is the recommended initial treatment for Guillain-Barré Syndrome (GBS)?

Management of Guillain-Barré Syndrome

First-Line Treatment

Intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days is the recommended first-line treatment for GBS patients who cannot walk unaided (GBS disability score ≥3), and should be initiated as early as possible, preferably within 2 weeks of symptom onset. 1, 2, 3

Why IVIg is Preferred Over Plasma Exchange

• IVIg and plasma exchange are equally effective in hastening recovery and reducing long-term morbidity in GBS 1, 4, 5
• IVIg is preferred as first-line therapy because it is easier to administer, more widely available, has higher completion rates (patients are significantly more likely to complete IVIg than PE), and causes fewer adverse effects 1, 2, 3, 4
• Plasma exchange remains a valid alternative, particularly in resource-limited settings where cost is a major factor (PE costs ~$4,500-5,000 vs IVIg ~$12,000-16,000), consisting of 5 sessions at 200-250 ml/kg total plasma volume over 1-2 weeks 6, 3

Treatment Timing and Indications

• Initiate treatment within 2 weeks of symptom onset for optimal efficacy, though treatment may still be beneficial up to 4 weeks for plasma exchange 1, 2, 5
• Treatment is indicated when patients cannot walk unaided (GBS disability score ≥3), or have moderate to severe weakness with rapid progression, respiratory compromise, dysphagia, facial weakness, or bulbar weakness 1, 2

Critical Monitoring Requirements

Respiratory Assessment: The "20/30/40 Rule"

Approximately 20% of GBS patients require mechanical ventilation, and close respiratory monitoring is essential 2, 3

• Patient is at high risk for respiratory failure if:

  • Vital capacity <20 ml/kg, OR
  • Maximum inspiratory pressure <30 cmH₂O, OR
  • Maximum expiratory pressure <40 cmH₂O 1, 2, 3

• Use the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to calculate probability of requiring ventilation 1, 5

• Admit patients to a unit with rapid transfer capability to ICU, as respiratory compromise can occur suddenly even during treatment 1

Neurological Monitoring

• Perform regular neurological assessments to monitor disease progression and treatment response, including motor strength, reflexes, and bulbar symptoms 1
• Monitor rigorously during and after each IVIg infusion for potential adverse reactions 1

Autonomic Dysfunction and Other Complications

• Monitor for autonomic dysfunction including cardiac arrhythmias, blood pressure fluctuations, and urinary retention 1, 5
• Assess for pain, which is common and requires aggressive management 1, 3
• Address constipation/ileus, which frequently occurs in GBS patients 1

Medications to AVOID

The following medications worsen neuromuscular function and must be avoided in GBS patients:

• β-blockers 1, 2, 3
• Aminoglycosides 2, 3
• IV magnesium 1, 2
• Fluoroquinolones 1, 2
• Macrolides 1, 2

Essential Supportive Care

Pain Management

• Use gabapentinoids (gabapentin, pregabalin), tricyclic antidepressants, duloxetine, or carbamazepine for neuropathic pain 1, 2, 5
• Avoid opioids for neuropathic pain management 1

Prevention of Complications

• Provide DVT prophylaxis due to immobility 1, 2, 3
• Implement pressure ulcer prevention through regular repositioning 1, 3
• Prevent hospital-acquired infections (pneumonia, UTIs) 3
• Evaluate for dysphagia and provide nutritional support if necessary 1

Special Populations

Children

• IVIg is strongly preferred over plasma exchange in children (0.4 g/kg/day for 5 days) due to better tolerability and fewer complications 1, 2, 3
• Use the standard 5-day regimen rather than accelerated 2-day protocols, as treatment-related fluctuations occur more frequently with shorter regimens 1

Pregnant Women

• IVIg is preferred over plasma exchange during pregnancy because it requires fewer monitoring considerations and additional precautions, though neither treatment is contraindicated 1, 2

Miller-Fisher Syndrome

• Treatment is generally not recommended for MFS, as most patients recover completely within 6 months without intervention, though close monitoring is essential 1

Immune Checkpoint Inhibitor-Related GBS

• Discontinue the causative agent permanently and consider concurrent corticosteroids with IVIg or plasma exchange 1, 2

Management of Treatment Non-Response and Fluctuations

Expected Timeline

• 40% of patients do not improve in the first 4 weeks following treatment—this does NOT necessarily indicate treatment failure 1, 3
• Recovery can continue for more than 5 years after disease onset 3

Treatment-Related Fluctuations (TRFs)

• TRFs occur in 6-10% of patients within 2 months of initial improvement 1, 3
• Repeat the full course of IVIg or plasma exchange for TRFs 1

Second Course of IVIg

• A second IVIg course is NOT recommended in GBS patients with a poor prognosis based on current evidence 5

Combination Therapy

• Do NOT give plasma exchange followed immediately by IVIg, as this does not confer significant extra benefit (mean grade improvement 0.2,95% CI -0.14 to 0.54) 4, 5

Treatments NOT Recommended

Corticosteroids

• Oral corticosteroids are NOT recommended for GBS treatment, as randomized controlled trials have shown no significant benefit and may even have negative effects on outcomes 1, 5
• IV corticosteroids are weakly recommended against 5

Prognostic Information

Recovery Expectations

• 80% of patients regain walking ability at 6 months after disease onset 1, 2, 3
• Mortality is 3-10%, most commonly from cardiovascular and respiratory complications 1, 2, 3

Risk Factors for Poor Outcome

• Advanced age 1, 3
• Severe disease at onset 1, 3
• Lack of ICU support when needed 3

Long-Term Sequelae

• Many patients have residual complaints including neuropathic pain, weakness, and fatigue that may persist for months or years 3, 7
• Recurrence is rare (2-5%) but higher than general population lifetime risk (0.1%) 3

Prognostic Tools

• Use the modified Erasmus GBS Outcome Score (mEGOS) to assess outcome 5
• Use mEGRIS to assess risk of requiring artificial ventilation 1, 5

Differential Diagnosis Considerations

If progression continues after 8 weeks from onset, consider changing the diagnosis to acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP), which occurs in approximately 5% of patients initially diagnosed with GBS 5, 7

Pre-Treatment Precautions

• Verify serum IgA levels before the first IVIg infusion, as IgA deficiency increases the risk of anaphylaxis, and use preparations with reduced IgA levels if deficiency is confirmed 1